Pulmonary epithelioid hemangioendothelioma: A rare pulmonary tumor in differential diagnosis of bronchogenic carcinoma

Darbari, Anshuman; Singh, Devender; Singh, Prashant Kumar; Bharadwaj, Manu

doi:10.4103/0970-2113.59268

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…Pulmonary epithelioid hemangioendothelioma (PEH) is the current term for a rare neoplasm originally described by Dail and Liebow in 1975 as intravascular sclerosing bronchioalveolar tumor (IVBAT) of the lung ( 1 , 2 ). PEH is a rare pulmonary neoplasm of vascular origin with fewer than 50 cases reported in the literature ( 3 – 6 ). PEH typically manifests as multiple bilateral lung nodules that are usually discovered incidentally in young or middle-aged Caucasian women, although cases in children and the elderly have also been reported ( 4 , 7 ).…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Shao

Zhang

2014

Oncology Letters

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This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25–54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

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Section: Introductionmentioning

confidence: 99%

Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Shao

Zhang

2014

Oncology Letters

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Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma

Zheng¹,

Wang²,

Jiang

et al. 2017

Medicine

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Rationale:Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare tumor, with no established standard treatment. Overexpression of vascular endothelial growth factor receptor 2 (VEGFR-2) has been reported in some P-EHE patients. Apatinib, a new small molecule tyrosine kinase inhibitor that specifically targets VEGFR-2, has therapeutic benefits in some advanced tumors. However, its efficacy in P-EHE cases has not been reported.Patient concerns:Herein, we presented a 44-year-old man with recurrent hemoptysis for approximately 9 years.Diagnoses:After hospitalization, relevant examinations were conducted. The disease was subsequently diagnosed as P-EHE.Interventions:The patient underwent pulmonary lobectomy, but subsequently developed multiple metastases. Within the tumor, CD31, CK, and Vimentin were found to be positive, while CD34 was negative. Apatinib was initially administered 250 mg daily doses and after 1 month was increased to 500 mg daily.Outcomes:He showed noticeable symptomatic improvements and positive imaging changes in the first month of treatment. However, the disease progressed in the following month, despite the increased apatinib dose.Lessons:Apatinib is possibly a new treatment for P-EHE. However, further clinical trials are necessary to confirm an effective dose and the efficacy and safety of apatinib in P-EHE treatment.

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Primary pulmonary epithelioid hemangioendothelioma

Jang,

Hung,

et al. 2023

BMJ Case Rep

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Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumour of vascular origin with low to intermediate malignancy. Typical radiological finding on CT is multiple small nodules in bilateral lungs, and some will have punctate calcifications and pleural thickening. The diagnosis of PEH is confirmed by histopathological findings and positive immunohistochemistry staining. We report a case of a woman in her 50s with a medical history of lung adenocarcinoma. Later, regular chest CT during a routine cancer follow-up revealed multiple small pulmonary nodules and increased sizes of these nodules on serial images, initially misdiagnosed as multiple lung metastases. The histopathological diagnosis was made on a pulmonary wedge resection. Finally, PEH was diagnosed on the basis of positive immunohistochemical staining for CD31, ERF and TFE3. In the current study, the clinicopathological features and review of the literature were investigated. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.

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Pulmonary epithelioid hemangioendothelioma: A rare pulmonary tumor in differential diagnosis of bronchogenic carcinoma

Cited by 3 publications

References 4 publications

Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma

Primary pulmonary epithelioid hemangioendothelioma

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